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Behcet's disease is the main clinical symptoms

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Updated: Saturday, Feb 20,2010, 3:06:01 PM
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Behcet's disease is a systemic, chronic, inflammatory vascular disease, the main clinical manifestations of recurrent oral ulcers, genital ulcers, ophthalmia and skin damage, but also can affect blood vessels, nervous system, gastrointestinal tract, joints, lungs, kidneys , epididymis and other organs, most patients with good prognosis, eyes, central nervous system and large vessel involvement were poor prognosis.

The disease in East Asia, the Middle East and the Mediterranean region have a higher prevalence, known as the Silk Road disease. No precise information on the incidence rate in China, of any age may be sick, good hair age of 16 to 40 years. China's women than men, male patients with blood vessels, nervous system and eye involvement and more severe illness than women.

Clinical manifestations 【】

The disease can be systemic involvement of various systems, but less at the same time that the problem of clinical manifestations. Sometimes patients had to go through several years or even longer, appearing in a variety of clinical symptoms and signs.

1, oral ulcers

Almost all patients had recurrent, painful oral ulcers (Aphthous ulceration, aphthous ulcers), most patients with this disease as the first symptom. Ulcers can occur in any part of the oral cavity, multi-lingual edge is located, cheek, lips, soft palate, pharynx, tonsil, etc.. For a single hair can also occur in batches, showing the size of a grain of rice or soya bean, round or oval, the edge clearly different shades of the bottom with yellow cover, surrounded by a clear and flush the edge, about 1 ~ 2 weeks after the self-limiting without leaving a scar. Persons with severe deep ulcers healed slowly, and even be a scar left behind. Recurrent oral ulcers are the most basic essential diagnostic symptoms of this disease.

2, genital ulcers

About 75% of patients with genital ulcers, lesions and oral ulcers similar. However, fewer occurrences. Deep ulcers, pain, drama, healing slow. Involvement sites were the vulva, vagina, anal, cervical, scrotum and penis, etc.. Vaginal secretions ulcer can be no pain only increased. There may be a result of ulcers in patients with deep vein Erzhi hemorrhage or necrosis of the scrotum bleeding.

3, ophthalmia

About 50% of the patients involved, involving both eyes. After the onset of ocular lesions can be after a few months or even years, its performance for the blurred vision, vision loss, eye hyperemia, eye pain, photophobia, tearing, foreign body sensation, Floaters, and headache. Usually presents with chronic, recurrent, progressive course of the disease. Eye involvement blindness rate of up to 25%, which is the main reason for disability of this disease. The most common and most serious ocular lesions and uveitis (uveitis). Anterior uveitis or iridocyclitis, may be associated with or without hypopyon, and then uveitis and retinitis is the main reason affecting eyesight. The rest of the eye may be involved organizations, there keratitis, herpetic conjunctivitis, scleritis, choroiditis, retinitis, optic nerve papillitis, necrotizing retinal vasculitis, retinal haemorrhage. In addition, the lens may have bleeding or atrophy, glaucoma, retinal detachment. Individual optic disc edema prompted cerebral venous thrombosis, caused by Behcet's disease with intracranial vascular lesions can lead to visual field defect.

4, skin lesions

A high incidence of lesions up to 80% ~ 98%, performance varied, with erythema nodosum, herpes, pimples, acne-like rash, erythema multiforme, annular erythema, necrotizing rash tuberculosis lesions, bullous necrotizing vasculitis, Sweet disease-like skin lesions, purulent skin diseases. A patient may have one or more of the lesions. The diagnostic value of particular signs of a nodular skin lesions and a tiny red spot kind of trauma (acupuncture), after the inflammatory response.

   5, joint damage

25% ~ 60% of patients with joint symptoms. Showed the limitations of the relatively mild, non-symmetrical arthritis. Mainly involving the knee and other large joints. HLA-B27 positive patients with sacroiliac joint involvement may have appeared similar to the performance and ankylosing spondylitis.

6, nervous system damage

Neuro-Behcet's disease, the incidence rate of about 5% ~ 50%. Frequently used in patients a few months to several years after the emergence of a few (5%) for the first symptom. Clinical manifestations vary according to site involved. The central nervous system involvement were more common, may have headache, dizziness, Horner syndrome, pseudobulbar palsy, respiratory disorder, epilepsy, ataxia, aseptic meningitis, papilloedema, hemiplegia, aphasia, varying degrees of paraplegia , urinary incontinence, double lower limb weakness, sensory disturbances, disturbance of consciousness, mental disorders. Peripheral nerve involvement is less common, manifested as limb numbness weakness, peripheral sensory disorders.

Nervous system damage is also a tendency to alternately attack and mitigation, which can more parts involved, the majority of patients with poor prognosis, particularly in the brain stem and spinal cord lesions of this disease one of the major disability and death.

7, gastrointestinal damage

Also known as intestinal Behcet's disease. Incidence rate of 10% ~ 50%. From the mouth to the anus can be involved the whole digestive tract, ulcers can be single or multiple, different shades, can be found in the lower esophagus, stomach, distal ileum, ileocecal, ascending colon, but more common ileocecal . Clinically manifested as epigastric fullness, belching, swallowing difficulties, abdominal fullness, pain, paroxysmal cramps, diarrhea, melena, constipation and so on. Severe cases may have ulcer perforation, or even died because of complications such as bleeding. Intestinal Behcet's disease should be noted that with inflammatory bowel disease and non-steroidal anti-inflammatory drugs induced differentiated from mucosal lesions, the right lower abdominal pain should be differentiated from appendicitis attention in clinical practice often have cases of non-healing wound.

8, vascular damage

The basic lesion of this disease as a vasculitis, involving both the body size of the vessel, about 10% ~ 20% of Patients with large and medium vessel vasculitis, is the main reason for death and disability. Arterial system is tired and timely destruction of arterial wall elastic fibers and arterial wall intimal fibrosis, resulting in arterial stenosis, aneurysm expansion or generated, a corresponding clinical manifestations may have dizziness, headache, syncope, no pulse. Aortic arch and its branches are ruptured aneurysm on the risk. Venous system involvement more common than the arterial system. About 25% of patients in the Department of superficial or deep migratory thrombophlebitis and venous thrombosis, resulting in stenosis and thrombosis. The inferior vena cava and lower extremity venous involvement more, there may be Budd-Chiari syndrome, ascites, lower limb edema. Superior vena cava obstruction may have facial, neck swelling, upper extremity venous pressure is increased.

9, lung damage

A lower incidence of lung damage, about 5% ~ 10%, but most of them seriously ill. Pulmonary vascular involvement may have a pulmonary artery aneurysm formation, can develop when the tumor ruptured pulmonary artery - bronchial fistula caused by pulmonary hemorrhage; pulmonary vein thrombosis can cause pulmonary embolism; alveolar capillary endothelial hyperplasia of fibrosis around the inflammation can affect the ventilation function. Pulmonary involvement when patients have cough, hemoptysis, chest pain, breathing difficulties. A large number of hemoptysis can cause death.

10 Other

Kidney damage is less, may have intermittent or persistent proteinuria or hematuria, renal hypertension and kidney checks may have IgA mesangial proliferative lesions or facial paralysis facial paralysis 9.

Less cardiac involvement, may have myocardial infarction, valvular disease, conduction system involvement, pericarditis, etc.. Intracardiac mural thrombosis may have a small number of patients showed expansion of the heart-like change, like the performance of constrictive pericarditis, heart disease and local changes related to vasculitis.

Epididymitis occurrence rate of about 4% ~ 10%, compared with specificity. Acute onset, expressed as a single or bilateral epididymal swelling pain and tenderness, 1 to 2 weeks may be alleviated, easy to recur.

The majority of patients sicker during pregnancy can also ease eye uveitis reported. May have intrauterine growth retardation, post-natal illness most aggravating. Nearly 10% of patients with fibromyalgia syndrome-like manifestations, women more common.

Tags: disease Behcet

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